Reactome | Mitochondrial BTD hydrolyses BCTN

Mitochondrial BTD hydrolyses BCTN

Stable Identifier

R-HSA-4167509

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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Human biotinidase (BTD, EC 3.5.1.12) (Cole et al. 1994) catalyzes the hydrolysis of biocytin (BCTN, aka biotinyl-lysine), a product of biotin dependent carboxylase degradation, to biotin (Btn) and lysine. As a result, Btn is again available to be used in the biotinylation of apo-carboxylases in the mitochondrion. BTD is both secreted from various cells and localised in the mitochondria (Wolf & Jensen 2005). BTD deficiency, an autosomal recessive disorder, results in a secondary Btn deficiency that leads to juvenile onset multiple carboxylase deficiency (MIM:253260) (Wolf et al. 1983).

Literature References

PubMed ID	Title	Journal	Year
16150625	Evolutionary conservation of biotinidase: implications for the enzyme's structure and subcellular localization Wolf, B, Jensen, K	Mol. Genet. Metab.	2005
7509806	Human serum biotinidase. cDNA cloning, sequence, and characterization Hymes, J, Wolf, B, Denson, T, Cole, H, Spence, JE, Lockyer, JM, Reynolds, TR, Buck, GA	J. Biol. Chem.	1994
6883721	Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency Wolf, B, Allen, RJ, Goodman, SI, Grier, RE, Kien, CL	Clin. Chim. Acta	1983